Predicting the severity of congenital high airway obstruction syndrome

Roybal, Jessica L.; Liechty, Kenneth W.; Hedrick, Holly L.; Bebbington, Michael; Johnson, Mark P.; Coleman, Beverly G.; Adzick, N. Scott; Flake, Alan W.

doi:10.1016/j.jpedsurg.2010.01.022

Cited by 67 publications

(89 citation statements)

References 28 publications

(30 reference statements)

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“…Cardiac compression and mediastinal shift occurs as the lungs enlarge. Increased intrathoracic pressure causes decreased venous return, cardiac failure and ascites 3 . This sequence explains the characteristic ultrasound features of large echogenic lungs, a flattened or inverted diaphragm, fluid filled dilated trachea and bronchi, mediastinal shift/compression of the heart, and fetal ascites or hydrops 1 …”

Section: Discussionmentioning

confidence: 98%

“…Fraser syndrome, an autosomal recessive disorder, is characterised by laryngeal atresia, cryptophlathmos, syndactyly, and urogenital defects 3 . The VACTERL association (vertebral anomalies, anal atresia, cardiac anomalies, tracheoesophageal fistula/oesophageal atresia, renal and limb anomalies)and TACRD association (tracheal atresia/laryngeal atresia, cardiac and renal anomalies, and duodenal atresia) are also considerations when CHAOS is suspected 3 …”

Section: Discussionmentioning

confidence: 99%

“…EXIT procedures have been reported in CHAOS with reasonable neonatal survival rates 3 5 , 8 These children, however, may remain tracheostomy dependent, require complicated staged airways reconstruction, are delayed in oral feeding and have speech limitations 3 . The ability to provide effective therapy relies on early and accurate prenatal diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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Congenital high airways obstruction syndrome – first and second trimester diagnosis

Paoletti

Robertson

2014

Australas J Ultrason Med

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Congenital High Airways Obstruction Syndrome (CHAOS) is a rare condition associated with high fetal or neonatal mortality. Prenatal diagnosis in the second trimester is made by the typical ultrasound features of large echogenic lungs, a flattened or inverted diaphragm, and fetal ascites or hydrops. We present two cases diagnosed at our institution; one in the second trimester, and a first trimester diagnosis. To the best of our knowledge, first trimester diagnosis of CHAOS has not been previously reported.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Congenital high airways obstruction syndrome – first and second trimester diagnosis

Paoletti

Robertson

2014

Australas J Ultrason Med

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“…Tracheal atresia is a very rare congenital malformation which takes place by deficient recanalization of the upper airways around the 10th week of gestation resulting in a clinical spectrum defined as congenital high airway obstruction syndrome (CHAOS) [1]. CHAOS is a rare abnormality, usually with a lethal outcome.…”

Section: Discussionmentioning

confidence: 99%

“…This clinical condition was brought into notice firstly by Hedrick in the late 1900s [1]. The true incidence of CHAOS is unknown.…”

Section: Introductionmentioning

confidence: 99%

Prenatal ultrasonography findings of fetus with congenital high airway obstruction (chaos): A case report and review of literature

Ekmekçi¹,

Gençdal²,

Kiziltug³

2017

Clin Obstet Gynecol Reprod Med

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Congenital high airway obstruction syndrome (CHAOS) is an extremely rare life-threatening condition. The sonographic findings are very characteristic. Generally the diagnosis is made with the 4-chamber view of the fetal heart. Typically, both lungs appear severely enlarged and highly echogenic. The heart points towards the midline of the thorax. Fetal ascites is determined commonly on ultrasound examination. A case of CHAOS, diagnosed antenatally via ultrasound is reported here.

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Congenital high airway obstruction sequence (CHAOS): A new case and a review of phenotypic features

Sanford

Saadai

Lee

et al. 2012

American J of Med Genetics Pt A

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Congenital high airway obstruction sequence (CHAOS) has traditionally been defined as airway obstruction with ultrasound evidence of distal airway dilatation, expanded lungs, ascites, and hydrops. It can result from aplasia or intrinsic obstruction to the formation of the upper airway (larynx and trachea) during development. CHAOS is commonly sporadic and there is no known causative gene. In this comprehensive review on CHAOS, we examined 117 reported cases of this sequence and describe a new case. Malformations in addition to high airway obstruction were present in 64/118 (54.2%) of patients. The most frequent anomalies affected the digits and musculoskeletal system, but there was no distinct phenotype or characteristic dysmorphic appearance associated with CHAOS. The ex utero intrapartum therapy (EXIT) procedure has greatly improved survival for these patients and 36/118 (30.5%) were alive at the time of reporting. Only 2 patients out of 12 who underwent laryngotracheoplasty were tracheostomy-free at the time of their reporting. Six out of 13 were able to produce some speech. Our review provides valuable information on associated anomalies and survival in this complex sequence. The phenotypic variability seen in this review of patients makes it likely that the causes of CHAOS are genetically heterogeneous.

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Predicting the severity of congenital high airway obstruction syndrome

Cited by 67 publications

References 28 publications

Congenital high airways obstruction syndrome – first and second trimester diagnosis

Congenital high airways obstruction syndrome – first and second trimester diagnosis

Prenatal ultrasonography findings of fetus with congenital high airway obstruction (chaos): A case report and review of literature

Congenital high airway obstruction sequence (CHAOS): A new case and a review of phenotypic features

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